Wernicke-Korsakoff Syndrome (WKS): Symptoms, Causes, Diagnosis And Treatment

Wernicke-Korsakoff syndrome, often abbreviated WKS, is a form of dementia that often goes undiagnosed among homeless people, older people who live alone, and long-term residents of psychiatric institutions. Autopsies indicate that it affects as much as three percent of the population, but the people who have it often are isolated and untreated.

One Syndrome, Two Diseases

Wernicke-Korsakoff Syndrome is one set of symptoms that is caused by two consecutive diseases. WSK presents with confusion, ataxia (stumble-drunk motions), and nystagmus (uncontrollable repetitive movements of the eyes). The first disease state in WSK is Wernicke encephalopathy. This treatable condition is caused by vitamin B₁ (thiamine) deficiency. People at risk for B₁ deficiencies include:

• Alcoholics. Chronic alcohol abuse leads to liver disease, which disables an enzyme the liver uses to activate vitamin B₁ into a form the body can use.
• Homeless people who eat lots of high-carbohydrate foods and who cannot access balanced meals.
• People who have had weight loss (bariatric) surgery. Wernicke encephalopathy can occur as quickly as two weeks after surgery, although recovery takes just a few weeks after supplementation begins.
• Pregnant women who have severe morning sickness. Wernicke encephalopathy often causes loss of the baby.
• People who have had tummy tucks (a plastic surgery also known as panniculectomy) go very wrong.
• People undergoing starvation. These are most commonly people with cancer or people who have schizophrenia.
• Babies given formula without added thiamine and babies fed breast milk from mothers who are themselves deficiency in thiamine.
• People who have anorexia nervosa. These people do not consume enough thiamine.
• People who have bowel obstructions or short bowel syndrome. These people cannot absorb enough thiamine.
• Prisoners of war. Their food may be lacking in thiamine. Famously, a group of prisoners of war held in Japan and given only white rice to eat developed WSK and they were among the first patients ever treated with intravenous B₁ and/or thiamine tablets. The IV B₁ was much more successful.

The body stores about an 18-day supply of thiamine. If thiamine is absent from the diet for 18 days, the brain is unable to make enzymes for the areas that have the highest metabolic activity. The blood-brain barrier may break down so that infections and certain toxic chemicals can enter the brain.

Initial symptoms of WSK

The phase of the disease known as Wernicke encephalopathy can manifest a variety of symptoms. Due to decreased energy production in the brain, there can be:

• Eye symptoms. There may be diplopia (double vision), strabismus (crossed eyes or wall eyes, eyes looking up or down), and other painless vision abnormalities.
• Gait abnormalities. These can include wide stance but short steps.
• Altered mental status. There may be hallucinations, confabulations (the patient fills in gaps in memory by making up a story), agitation, or apathy.

There may also be weight loss and other problems caused by deficiencies of iron and/or vitamin B₁₂.

This phase of the disease can be treated with intravenous thiamine. Vitamin B₁ supplement pills are usually not sufficiently well absorbed because the body cannot make the enzymes needed to absorb and activate them. IV B₁ is given as long as improvements are noted.

Korsakoff syndrome

If treatment is delayed, Wernicke's encephalopathy may progress to a form of dementia known as Korsakoff syndrome. These patients need long-term hospital care. When they recover, there is a predictable pattern of improvement:

• Eye abnormalities. This first phase of recovery is usually dramatic, and may occur within just a few hours of the first dose of thiamine. Strabismus stops first, and nystagmus follows over the next few months.
• Ataxia complications. About 40 percent of people who develop Korsakoff dementia recover from their slow, shuffling walk and their wide stance for standing. 
• Global confusion state. Only about 20 percent of people who develop Korsakoff dementia with amnesia recover normal mental status. Improvement takes at least a year and depends on absolute abstinence from alcohol.

If someone in your family has WSK

Taking care of someone who has WSK is a full-time job. If you don't have access to home health care, you may have to consider an institutional placement for your loved one who has WSK.

Unassisted walking is not recommended during the first few months of treatment, due to problems with gait. WSK patients need a well-balanced diet with adequate fluid and electrolytes. It is important not to have alcohol in their living quarters because they usually lack the insight and impulse control not to drink it.

High-carbohydrate diets increase the body's demands for thiamine. It is best to avoid sweets and sugar as long as the patient is still receiving thiamine injections or high-dose thiamine supplements.